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Persistent morbidity and mortality of protein calorie malnutrition in young infants with CF
Insights
Young infants with cystic fibrosis (CF) can develop severe malnutrition (PCM). Early diagnosis and pancreatic enzyme therapy are crucial for preventing this serious condition in infants with CF.
Area of Science:
- Pediatrics
- Gastroenterology
- Pulmonology
Background:
- Infants with cystic fibrosis (CF) are susceptible to protein-calorie malnutrition (PCM).
- PCM in CF presents with hypoalbuminemia, edema, and anemia.
- This condition poses a significant risk for morbidity and mortality in young CF patients.
Purpose of the Study:
- To investigate the incidence and characteristics of symptomatic protein-calorie malnutrition (PCM) in infants under 12 months with cystic fibrosis (CF).
- To identify risk factors associated with PCM development in this vulnerable population.
- To underscore the importance of early diagnosis and management of CF in preventing PCM.
Main Methods:
- Retrospective review of hospital charts for infants under 12 months diagnosed with CF between 1979 and 1982.
- Analysis of clinical data including age at presentation, feeding methods, presenting symptoms, respiratory status, infections, and treatment with pancreatic enzymes.
- Identification of patients who developed symptomatic PCM.
Main Results:
- Nine out of 70 infants (13%) presented with PCM.
- Common factors included young age (under 7 months), soy formula use, and pre-diagnosis gastrointestinal or respiratory symptoms.
- High mortality (5/9) was observed, particularly in infants not receiving pancreatic enzyme therapy.
Conclusions:
- Protein-calorie malnutrition (PCM) remains a significant cause of morbidity and mortality in young infants with cystic fibrosis (CF).
- Soy formula feeding and lack of pancreatic enzyme supplementation are identified as key risk factors.
- Early diagnosis of CF and prompt initiation of pancreatic enzyme therapy may reduce the incidence and severity of PCM in infants.
Abstract:
Young infants with cystic fibrosis (CF) are at risk for developing symptomatic protein-calorie malnutrition (PCM) characterized by hypoalbuminemia, edema, and anemia. We reviewed the hospital charts of all infants less than 12 months of age referred to our CF clinic between 1979 and 1982 and found nine patients with PCM (13%). Their courses were characterized by age less than 7 months (nine patients), frequent use of soy formula (eight patients), presence of gastrointestinal or respiratory symptoms before being diagnosed with CF (eight patients), severe respiratory distress often requiring mechanical ventilation (five patients), significant infections (three patients), and high mortality (five patients). We further noted that eight infants were not on pancreatic enzyme therapy at the onset of PCM. This clinical study extends the findings of earlier reports of PCM in patients with CF by showing that this syndrome persists as a major source of morbidity and mortality in young infants with CF, especially those fed soy formula and not receiving pancreatic enzyme supplements. Because PCM can develop rapidly and may be the presenting sign of CF in young infants, we speculate that early diagnosis of CF with institution of pancreatic supplements may decrease incidence and severity of PCM in young patients with CF.